Paraneoplastic Disorders in Thymoma Patients

Evoli, Amelia; Lancaster, Eric

doi:10.1097/jto.0000000000000300

Cited by 113 publications

(89 citation statements)

References 46 publications

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“…A high level of GluR1/2 and GluR2/3 receptors is found in the synaptic CA3-CA1 areas of the hippocampus followed by the subiculum, cerebellum, caudate-putamen, and cerebral cortex [7]. The thymus is a central organ for the development of the immune system, particularly for the selection of T cells with appropriate self-tolerance [8]. It is not surprising that thymoma is considered to be a possible initiator for most paraneoplastic neurological disease, such as AE [8].…”

Section: Discussionmentioning

confidence: 99%

“…The thymus is a central organ for the development of the immune system, particularly for the selection of T cells with appropriate self-tolerance [8]. It is not surprising that thymoma is considered to be a possible initiator for most paraneoplastic neurological disease, such as AE [8]. In 2009, Lai [7] first reported three patients with anti-AMPAR encephalitis, and thymoma were identified among 109 cases of limbic encephalitis.…”

Section: Discussionmentioning

confidence: 99%

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Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR2 encephalitis in a myasthenia gravis patient with complete thymectomy: a case report

2019

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Background: Autoimmune encephalitis (AE) is a newly recognized autoimmune disorders in which the targets are proteins or receptors involved in synaptic transmission and neuronal excitability. α-amino-3-hydroxy-5-methyl-4isoxazolepropionic acid receptor (AMPAR) is a subtype of glutamate receptor that mediates most of the fast excitatory neurotransmission in the brain. Case presentation: A 50-year-old woman presented with subacute onset of memory loss and behavioral changes. High levels of serum (1:1000) and CSF (1:32) antibodies against the AMPAR GluR2 were detected. A wide range of abnormalities in 6-8 Hz low to middle slow waves was found by electroencephalographs, and high-intensity signals on fluid-attenuated inversion recovery in both the medial temporal lobe and hippocampus were identified on brain magnetic resonance images. This patient presented with myasthenia gravis and type B2 thymoma (World Health Organization Thymoma Classification) at age 48. This case was unique in that the patient initiated with the symptom of myasthenia gravis and thymoma two years prior to encephalitis, and a complete thymectomy was performed before AE onset without recurrence of the thymoma when encephalitis occurred. Conclusions: Thymoma was reported to be associated with paraneoplastic neurological disease. This is the first time a thymectomy has been applied in a myasthenia gravis patient with thymoma two years prior to the onset of anti-AMPAR2 encephalitis. This case highlights the complexity of autoimmune encephalitis associated with thymoma.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR2 encephalitis in a myasthenia gravis patient with complete thymectomy: a case report

2019

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“…The association of SPS with thymoma probably reflects the propensity of this tumor to induce a variety of autoimmune disorders and circulating autoantibodies. 40,41 There is no evidence that thymoma cells express GAD65 or GAD67; therefore, the possible pathogenic mechanisms remain unclear. Patients with SPS and breast cancer usually harbor amphiphysin antibodies rather than GAD-abs.…”

Section: Discussionmentioning

confidence: 99%

Paraneoplastic Neurological Syndromes and Glutamic Acid Decarboxylase Antibodies

et al. 2015

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IMPORTANCE Little is known of glutamic acid decarboxylase antibodies (GAD-abs) in the paraneoplastic context. Clinical recognition of such cases will lead to prompt tumor diagnosis and appropriate treatment.OBJECTIVE To report the clinical and immunological features of patients with paraneoplastic neurological syndromes (PNS) and GAD-abs. DESIGN, SETTING, AND PARTICIPANTSRetrospective case series study and immunological investigations conducted in February 2014 in a center for autoimmune neurological disorders. Fifteen cases with GAD65-abs evaluated between 1995 and 2013 who fulfilled criteria of definite or possible PNS without concomitant onconeural antibodies were included in this study. MAIN OUTCOMES AND MEASURESAnalysis of the clinical records of 15 patients and review of 19 previously reported cases. Indirect immunofluorescence with rat hippocampal neuronal cultures and cell-based assays with known neuronal cell-surface antigens were used. One hundred six patients with GAD65-abs and no cancer served as control individuals.RESULTS Eight of the 15 patients with cancer presented as classic paraneoplastic syndromes (5 limbic encephalitis, 1 paraneoplastic encephalomyelitis, 1 paraneoplastic cerebellar degeneration, and 1 opsoclonus-myoclonus syndrome). When compared with the 106 non-PNS cases, those with PNS were older (median age, 60 years vs 48 years; P = .03), more frequently male (60% vs 13%; P < .001), and had more often coexisting neuronal cell-surface antibodies, mainly against γ-aminobutyric acid receptors (53% vs 11%; P < .001). The tumors more frequently involved were lung (n = 6) and thymic neoplasms (n = 4). The risk for an underlying tumor was higher if the presentation was a classic PNS, if it was different from stiff-person syndrome or cerebellar ataxia (odds ratio, 10.5; 95% CI, 3.2-34.5), or if the patient had coexisting neuronal cell-surface antibodies (odds ratio, 6.8; 95% CI, 1.1-40.5). Compared with the current series, the 19 previously reported cases had more frequent stiff-person syndrome (74% vs 13%; P = .001) and better responses to treatment (79% vs 27%; P = .005). Predictors of improvement in the 34 patients (current and previously reported) included presentation with stiff-person syndrome and the presence of a thymic tumor. CONCLUSIONS AND RELEVANCEPatients with GAD-abs must be screened for an underlying cancer if they have clinical presentations different from those typically associated with this autoimmunity or develop classic PNS. The risk for cancer increases with age, male sex, and the presence of coexisting neuronal cell-surface antibodies.

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“…This is consistent with previous reports outlining the co-occurrence of these syndromes with thymoma. 27 Other autoimmune diseases including Lambert-Eaton myasthenic syndrome, encephalitis, autonomic neuropathy, StiffPerson syndrome have also been reported with thymoma, with or without MG. [27][28][29][30] It can be speculated that specific antigens related to these illnesses are expressed in thymoma, leading to aberrant T cell priming and subsequent specific autoimmunity.…”

Section: Discussionmentioning

confidence: 96%

A Case Series of Patients With Autoimmune Myasthenia Gravis in Association With Invasive Thymoma

McCombe

Raj

Henderson³

et al. 2016

Journal of Clinical Neuromuscular Disease

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Paraneoplastic Disorders in Thymoma Patients

Cited by 113 publications

References 46 publications

Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR2 encephalitis in a myasthenia gravis patient with complete thymectomy: a case report

Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR2 encephalitis in a myasthenia gravis patient with complete thymectomy: a case report

Paraneoplastic Neurological Syndromes and Glutamic Acid Decarboxylase Antibodies

A Case Series of Patients With Autoimmune Myasthenia Gravis in Association With Invasive Thymoma

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